An Unusual Cause of Chronic Headache in an Adolescent Boy: A Case Report

Abstract

Spontaneous intracranial hypotension (SIH) is an under-diagnosed cause of headache in children and adolescents. SIH results from cerebrospinal fluid (CSF) leak due to breach in the dura mater and the etiology for dural breach is often diverse. We report an adolescent boy who presented with chronic episodic headache that later progressed to daily headache. There was a typical history of worsening of headache on upright position and relief of headache on lying down. He was treated with migraine prophylaxis in another hospital but there was no response. Marfanoid features and brisk deep tendon reflexes were observed on clinical examination. Brain magnetic resonance imaging (MRI) revealed sagging of the brain stem, pachymeningeal enhancement, and tonsillar herniation. MRI of spine myelogram confirmed multiple levels of CSF leak. He was initially managed with supportive measures and fluoroscopic-guided fibrin glue injection. Although child remained symptom-free for the next 6 months, he again developed headache. MRI and computed tomography spine myelogram revealed a meningeal diverticulum in the lumbar spine. He was managed with an autologous epidural blood patch and he has been well since then. In this report, we highlight the clinical and radiological pointers to the presence of SIH in children with recurrent headache.

Keywords

headache - spontaneous intracranial hypotension - CSF leak - epidural blood patch - fibrin glue injection

Author's Contribution

Sangeetha Yoganathan prepared the initial manuscript and Sniya Valsa Sudhakar prepared the figure. Sangeetha Yoganathan, Vivek Mathew, Maya Thomas, and Fouzia. N.A were involved in the clinical care of the patient. Sunithi Elizabeth Mani and Sniya Valsa Sudhakar established the radiological diagnosis. Sniya Valsa Sudhakar prepared the images. Ramamani Mariappan was involved in the management of the patient with fibrin glue injection and autologous epidural blood patch. All the authors revised the manuscript for its intellectual content and approved the final manuscript for submission.

Disclosure

This case was presented as a poster elaborating the Epidural Blood Patch technique in AIIMS Neuroanaesthesia Update- 2017, held in New Delhi, India.

• References

• 1 Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006; 295 (19) 2286-2296
• 2 Amoozegar F, Guglielmin D, Hu W, Chan D, Becker WJ. Spontaneous intracranial hypotension: recommendations for management. Can J Neurol Sci 2013; 40 (02) 144-157
• 3 Couch JR. Spontaneous intracranial hypotension: the syndrome and its complications. Curr Treat Options Neurol 2008; 10 (01) 3-11
• 4 The International Classification of Headache Disorders. 3rd edition (beta version). Headache Classification Committee of the International Headache Society (IHS). Cephalalgia 2013; 33: 629-808
• 5 Vetrugno R, Mascalchi M, Chierichetti F. , et al. Hypoactive-hypoalert behavior (“psychic akinesia”) in intracranial hypotension syndrome. Neurology 2008; 71 (18) 1452-1454
• 6 Walker L, DeMeulemeester C. Spontaneous intracranial hypotension masquerading as frontotemporal dementia. Clin Neuropsychol 2008; 22 (06) 1035-1053
• 7 Mokri B. Spontaneous intracranial hypotension. Curr Pain Headache Rep 2001; 5 (03) 284-291
• 8 Uysal S, Albayram S, Ercan TE. Spontaneous intracranial hypotension: a case report. J Child Neurol 2008; 23 (11) 1312-1315
• 9 Schievink WI. Spontaneous spinal cerebrospinal fluid leaks. Cephalalgia 2008; 28 (12) 1345-1356
• 10 Gordon N. Spontaneous intracranial hypotension. Dev Med Child Neurol 2009; 51 (12) 932-935
• 11 Pabaney AH, Mirza FA, Syed NA, Ahsan H. Spontaneous dural tear leading to intracranial hypotension and tonsillar herniation in Marfan syndrome: a case report. BMC Neurol 2010; 10: 54
• 12 Schievink WI, Gordon OK, Tourje J. Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. Neurosurgery 2004; 54 (01) 65-70 , discussion 70–71
• 13 Mokri B, Maher CO, Sencakova D. Spontaneous CSF leaks: underlying disorder of connective tissue. Neurology 2002; 58 (05) 814-816
• 14 Alvarez-Linera J, Escribano J, Benito-León J, Porta-Etessam J, Rovira A. Pituitary enlargement in patients with intracranial hypotension syndrome. Neurology 2000; 55 (12) 1895-1897
• 15 Schievink WI, Maya MM, Jean-Pierre S, Nuño M, Prasad RS, Moser FG. A classification system of spontaneous spinal CSF leaks. Neurology 2016; 87 (07) 673-679
• 16 Schievink WI, Maya MM, Louy C, Moser FG, Tourje J. Diagnostic criteria for spontaneous spinal CSF leaks and intracranial hypotension. AJNR Am J Neuroradiol 2008; 29 (05) 853-856
• 17 Scott S, Davenport R. Low pressure headaches caused by spontaneous intracranial hypotension. BMJ 2014; 349: g6219
• 18 Elwood JJ, Dewan M, Smith JM, Mokri B, Mauck WD, Eldrige JS. Efficacy of epidural blood patch with fibrin glue additive in refractory headache due to intracranial hypotension: preliminary report. Springerplus 2016; 5: 317
• 19 Schievink WI, Maya MM, Louy C. Cranial MRI predicts outcome of spontaneous intracranial hypotension. Neurology 2005; 64 (07) 1282-1284